TY - JOUR
T1 - A nation-wide survey of Japanese pediatric MOG antibody-associated diseases
AU - Azumagawa, Kohji
AU - Nakashima, Ichiro
AU - Kaneko, Kimihiko
AU - Torisu, Hiroyuki
AU - Sakai, Yasunari
AU - Kira, Ryutaro
AU - Sakuma, Hiroshi
AU - Tanaka, Keiko
AU - Shigeri, Yasushi
AU - Tanaka, Yoshie
AU - Nakajima, Hideto
AU - Shimakawa, Shuichi
AU - Tamai, Hiroshi
N1 - Funding Information:
The authors would like to thank all responders to the questionnaire and JSCN for their support of the investigation. We are also grateful to Dr. Leslie Sargent Jones (Appalachian State University, Retired) for her careful reading of our manuscript. No external funding for this manuscript. The authors have indicated they have no financial relationship relevant to this article to disclose.
Publisher Copyright:
© 2021 The Japanese Society of Child Neurology
PY - 2021/6
Y1 - 2021/6
N2 - Objective: To elucidate the clinical characteristics of Japanese pediatric patients with acquired demyelinating diseases (ADS), positive for myelin oligodendrocyte glycoprotein antibody (MOG-IgG), we conducted a nation-wide survey. Methods: Information about pediatric patients under 18 years old with ADS was solicited with surveys sent to 323 facilities. In an initial survey, we asked whether the center had any patients with ADS, and the MOG-IgG serostatus of the patients. In a follow-up survey, we requested more precise information on patients with ADS. Results: Initial survey: 263 replies providing information on 175 patients were received. MOG-IgG were examined in 78 patients and 54 of those (69%) were positive for MOG-IgG. Follow-up survey: The characteristic involvement was optic neuritis, with visual disturbance and optic pain as characteristic symptoms. The relapse rate was 44% in patients positive for MOG-IgG, which was higher than that in seronegative patients (38%). For acute phase treatments, corticosteroid (CS), plasma exchange, and intravenous immunoglobulin (IVIG) were useful. To prevent relapse, CS, intermittent IVIG, immunosuppressants, and monoclonal antibodies were useful, but the efficacies of disease modifying drugs were uncertain. Sequelae such as visual disturbance, cognitive impairment, motor dysfunction, and epilepsy were observed in 11% of patients with MOG-IgG. Conclusions: MOG antibody-associated diseases were found to be common among pediatric ADS patients. Since a variety of sequelae were observed in these patients, it is important to identify the appropriate treatment to ensure the best outcome. The presence of the MOG autoantibody should be taken into consideration as part of the diagnostic criteria for pediatric ADS.
AB - Objective: To elucidate the clinical characteristics of Japanese pediatric patients with acquired demyelinating diseases (ADS), positive for myelin oligodendrocyte glycoprotein antibody (MOG-IgG), we conducted a nation-wide survey. Methods: Information about pediatric patients under 18 years old with ADS was solicited with surveys sent to 323 facilities. In an initial survey, we asked whether the center had any patients with ADS, and the MOG-IgG serostatus of the patients. In a follow-up survey, we requested more precise information on patients with ADS. Results: Initial survey: 263 replies providing information on 175 patients were received. MOG-IgG were examined in 78 patients and 54 of those (69%) were positive for MOG-IgG. Follow-up survey: The characteristic involvement was optic neuritis, with visual disturbance and optic pain as characteristic symptoms. The relapse rate was 44% in patients positive for MOG-IgG, which was higher than that in seronegative patients (38%). For acute phase treatments, corticosteroid (CS), plasma exchange, and intravenous immunoglobulin (IVIG) were useful. To prevent relapse, CS, intermittent IVIG, immunosuppressants, and monoclonal antibodies were useful, but the efficacies of disease modifying drugs were uncertain. Sequelae such as visual disturbance, cognitive impairment, motor dysfunction, and epilepsy were observed in 11% of patients with MOG-IgG. Conclusions: MOG antibody-associated diseases were found to be common among pediatric ADS patients. Since a variety of sequelae were observed in these patients, it is important to identify the appropriate treatment to ensure the best outcome. The presence of the MOG autoantibody should be taken into consideration as part of the diagnostic criteria for pediatric ADS.
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U2 - 10.1016/j.braindev.2021.01.008
DO - 10.1016/j.braindev.2021.01.008
M3 - Article
C2 - 33610339
AN - SCOPUS:85101081693
SN - 0387-7604
VL - 43
SP - 705
EP - 713
JO - Brain and Development
JF - Brain and Development
IS - 6
ER -